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1.
Medicine (Baltimore) ; 99(6): e18978, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32028407

RESUMO

RATIONALE: AA amyloidosis (AA) is caused by a wide variety of inflammatory states, but is infrequently associated with Castleman disease (CD). CD describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. CD can present with a solitary enlarged lymph node (unicentric CD, UCD) or with multicentric lymphadenopathy (MCD), constitutional symptoms, cytopenias, and multiple organ dysfunction due to an interleukin-6 driven cytokine storm. PATIENT CONCERNS: We are reporting a case of a 26-year-old woman with no significant past medical history who presented with a 3-month history of fatigue and an unintentional 20-pound weight loss. DIAGNOSIS: A CT-scan of the abdomen and pelvis revealed hepatosplenomegaly and a mesenteric mass. Congo Red staining from a liver biopsy showed apple-green birefringence and serum markers were suggestive of an inflammatory process. Post-excision examination of the resected mass revealed a reactive lymph node with follicular hyperplasia with kappa and lambda stains showing polyclonal plasmacytosis consistent with CD. INTERVENTIONS: The patient underwent surgery to remove the affected lymph node. OUTCOMES: IL-6, anemia, leukocytosis, and thrombocytosis resolved or normalized 2 weeks after resection; creatinine normalized 9 months postsurgery. Twenty two months post-surgery her IFN-γ normalized, her fatigue resolved, her proteinuria was reduced by >90% and she had returned to her baseline weight. LESSONS: Our case and literature review suggest that patients presenting with UCD or MCD along with organ failure should prompt consideration of concurrent AA amyloidosis.


Assuntos
Amiloidose/etiologia , Hiperplasia do Linfonodo Gigante/complicações , Doenças Peritoneais/etiologia , Adulto , Amiloidose/sangue , Amiloidose/diagnóstico , Amiloidose/diagnóstico por imagem , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/diagnóstico , Fadiga/etiologia , Feminino , Humanos , Doenças Peritoneais/sangue , Doenças Peritoneais/diagnóstico , Doenças Peritoneais/diagnóstico por imagem , Proteína Amiloide A Sérica/análise , Tomografia Computadorizada por Raios X , Perda de Peso
2.
Medicine (Baltimore) ; 98(18): e15237, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31045763

RESUMO

RATIONALE: Idiopathic multicentric Castleman disease (iMCD) is a systemic disease with multiple regions of lymphadenopathy and systemic symptoms and associated with rheumatoid arthritis (RA) and collagen diseases. However, few reported have described the coexistence of iMCD and RA and the mechanisms by which iMCD induces arthritis remain elusive. We experienced a rare case of iMCD, wherein the patient exhibited symptoms of polyarthritis with high-grade fever. PATIENT CONCERNS: A 34-year-old woman was admitted to our hospital for further evaluation of a high fever with polyarthritis. The levels of both rheumatoid factor and anticitrullinated protein antibody were negative. F-fluorodeoxyglucose/positron emission tomography-computed tomography showed lymphadenopathy with increased fluoro-2-deoxy-D-glucose uptake. Magnetic resonance imaging and musculoskeletal ultrasonography revealed active synovitis in the hands which was consistent with RA. DIAGNOSES: We diagnosed iMCD based on human herpesvirus 8 negativity, HIV negativity, systemic lymphadenopathy, and pathologic findings of the lymph nodes. The patient did not satisfy the 2010 American College of Rheumatology and European League Against Rheumatism classification criteria for RA. Cytokine assay showed elevated serum levels of interleukin-17 and CXCL10, comparable to those in patients with RA. INTERVENTIONS: We administered 15 mg/d of predonisolone. OUTCOMES: After this treatment, the patient's symptoms showed improvement. As of this writing, we tapered the prednisolone to 7.5 mg/d, and the patient's remission has been maintained for >4 months. LESSONS: The present case suggests that RA-like active synovitis may coexist in iMCD, resulting from aberrant T-cell activation and histologic examination using lymph node biopsy may help enable early diagnosis of iMCD.


Assuntos
Artrite Reumatoide/complicações , Hiperplasia do Linfonodo Gigante/complicações , Linfadenopatia/diagnóstico por imagem , Sinovite/diagnóstico por imagem , Adulto , Artrite/diagnóstico , Artrite/etiologia , Artrite Reumatoide/diagnóstico por imagem , Artrite Reumatoide/patologia , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Hiperplasia do Linfonodo Gigante/patologia , Quimiocina CXCL10/sangue , Diagnóstico Diferencial , Feminino , Febre/diagnóstico , Febre/etiologia , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Herpesvirus Humano 8 , Humanos , Interleucina-17/sangue , Linfadenopatia/patologia , Imagem por Ressonância Magnética/métodos , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Sinovite/patologia , Linfócitos T/patologia , Resultado do Tratamento
3.
Acta Haematol ; 141(3): 158-163, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30799408

RESUMO

TAFRO syndrome, a rare systemic inflammatory disease, can lead to multiorgan failure without appropriate treatment. Although thrombocytopenia is frequently seen in patients with TAFRO syndrome, little is known about its pathogenesis. Moreover, while recent studies have reported the presence of an anterior mediastinal mass in some patients, the pathological status of this remains unclear. Here, we report a case of fatal bleeding in a patient with TAFRO syndrome accompanied by an anterior mediastinal mass. A 55-year-old female was transferred to our hospital with a 2-week history of fever, epistaxis, and dyspnea. Laboratory tests revealed severe thrombocytopenia, computed tomography (CT) showed pleural effusions, and bone marrow biopsy revealed reticulin myelofibrosis. We suspected TAFRO syndrome, but the CT scan showed an anterior mediastinal mass that required a biopsy to exclude malignancy. She soon developed severe hemorrhagic diathesis and died of intracranial hemorrhage despite intensive treatment. She had multiple autoantibodies against platelets, which caused platelet destruction. An autopsy of the mediastinal mass revealed fibrous thymus tissues with infiltration by plasma cells. Our case suggests that thrombocytopenia could be attributed to antibody-mediated destruction and could be lethal. Hence, immediate treatment is imperative in cases of severe thrombocytopenia, even when accompanied by an anterior mediastinal mass.


Assuntos
Autoanticorpos , Hiperplasia do Linfonodo Gigante , Doenças do Mediastino , Púrpura Trombocitopênica Idiopática , Tomografia Computadorizada por Raios X , Autopsia , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/diagnóstico por imagem , Hiperplasia do Linfonodo Gigante/patologia , Hiperplasia do Linfonodo Gigante/terapia , Evolução Fatal , Feminino , Humanos , Hemorragias Intracranianas/sangue , Hemorragias Intracranianas/diagnóstico por imagem , Hemorragias Intracranianas/patologia , Hemorragias Intracranianas/terapia , Doenças do Mediastino/sangue , Doenças do Mediastino/diagnóstico por imagem , Doenças do Mediastino/patologia , Doenças do Mediastino/terapia , Pessoa de Meia-Idade , Derrame Pleural/sangue , Derrame Pleural/diagnóstico , Derrame Pleural/patologia , Derrame Pleural/terapia , Mielofibrose Primária/sangue , Mielofibrose Primária/diagnóstico por imagem , Mielofibrose Primária/patologia , Mielofibrose Primária/terapia , Púrpura Trombocitopênica Idiopática/sangue , Púrpura Trombocitopênica Idiopática/diagnóstico por imagem , Púrpura Trombocitopênica Idiopática/patologia , Púrpura Trombocitopênica Idiopática/terapia
4.
Mod Rheumatol ; 29(2): 251-256, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30474465

RESUMO

IgG4-related diseases (IgG4-RDs), such as autoimmune pancreatitis and IgG4-related Mikulicz disease, are often accompanied by intrathoracic lesions, which are called IgG4-related respiratory disease (IgG4-RRD). IgG4-RRD has few subjective symptoms, and is usually detected during workup of patients with extra-thoracic lesions of IgG4-RD. IgG4-RRD is characterized by various conditions, including masses, nodules, thickening, and infiltration at numerous sites in the thorax through lymphatic routes. Although elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells are characteristic findings of IgG4-RD, other intrathoracic diseases, such as multicentric Castleman disease and malignancy, may present with similar findings. Developing diagnostic criteria for IgG4-RRD, including clinicoradiological and pathological characteristics, is necessary for its appropriate diagnosis.


Assuntos
Hiperplasia do Linfonodo Gigante/diagnóstico , Doença Relacionada a Imunoglobulina G4/diagnóstico , Pneumonia/diagnóstico , Biomarcadores/sangue , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/diagnóstico por imagem , Diagnóstico Diferencial , Humanos , Doença Relacionada a Imunoglobulina G4/sangue , Doença Relacionada a Imunoglobulina G4/diagnóstico por imagem , Pneumonia/sangue , Pneumonia/diagnóstico por imagem
5.
Br J Haematol ; 184(2): 232-241, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30203839

RESUMO

Siltuximab is the only US Food and Drug Administration-approved treatment for idiopathic multicentric Castleman disease (iMCD), a rare haematological disorder associated with substantial morbidity and mortality. Although siltuximab induces a response in a significant proportion of iMCD patients via interleukin 6 (IL6) neutralization, it is not universally effective. To develop a predictive model of response, we performed an in-depth analysis of 38 baseline laboratory parameters in iMCD patients from the phase II siltuximab trial who met criteria for treatment response or treatment failure. Univariate analyses identified eight baseline laboratory parameters that were significantly different between responders and treatment failures: albumin, immunoglobulin G (IgG), immunoglobulin A, C reactive protein (CRP), fibrinogen, haemoglobin, sodium and triglycerides. Stepwise logistic regression analysis of these candidate parameters identified a top performing model that included fibrinogen, IgG, haemoglobin and CRP. Based on cross-validation of the final multivariate logistic regression model, the model accurately discriminated responders from those who failed treatment (area under the receiver operator characteristic curve 0·86, 95% confidence interval: 0·73-0·95). All four laboratory parameters associated with response to siltuximab have biological relationships with IL6 and acute inflammation. Our model suggests that iMCD patients with laboratory evidence of an inflammatory syndrome are the best candidates for siltuximab therapy.


Assuntos
Anticorpos Monoclonais/administração & dosagem , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Interleucina-6/antagonistas & inibidores , Modelos Biológicos , Adulto , Anticorpos Monoclonais/efeitos adversos , Proteína C-Reativa/metabolismo , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/patologia , Feminino , Fibrinogênio/metabolismo , Humanos , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Interleucina-6/sangue , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes
7.
Clin Lab ; 64(10): 1671-1678, 2018 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-30336525

RESUMO

BACKGROUND: It can be difficult to distinguish between IgG4-related lymphadenopathy and multicentric Castleman's disease (MCD) because these conditions cannot be differentially diagnosed using immunohistochemical staining alone. In this study, we analyzed the clinical features of IgG4-related lymphadenopathy and MCD patients. METHODS: We retrospectively analyzed 27 patients with MCD, including 20 with plasma cell-type (PC-type) and 7 with hyaline vascular (HV) features (mixed-type). An additional 15 patients with IgG4-related lymphadenopathy were enrolled. Clinical data and immune pathological characteristics, including serum interleukin-6 (IL-6) levels, lymph node lesion biopsies, IgG4+/IgG+ expression, and 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) images, were collected. RESULTS: The serum levels of C-reactive protein (CRP), IgA, and IL-6 were significantly elevated in the PC/mixedtype group compared with the IgG4-related lymphadenopathy group (p < 0.05). By contrast, the mean age, eosinophilia, globulin, and serum levels of IgG and IgG4 were significantly higher in the IgG4-RD lymphadenopathy group (all p < 0.05). Thirty percent of patients with IgG4-RD lymphadenopathy had elevated IL-6 levels, and 50% with MCD had elevated serum IgG4 levels. Immunohistochemical studies demonstrated the presence of numerous IgG4+ plasma cells, which accounted for > 40% of IgG4/IgG+ cells in 7 of 27 cases in the PC/mixed-type group. We first found that the mean maximum standard uptake value (SUVmax) was strongly associated with albumin and IL-6 in the IgG4-RD lymphadenopathy group, but not in the MCD group. The number of involved organs, but not the standard uptake value (SUV), helped to distinguish between the two diseases. Most PC/mixed-type group patients responded poorly to glucocorticoids when administered alone or in combination with immunosuppressant drugs. CONCLUSIONS: MCD cannot be differentiated from IgG4-related lymphadenopathy using histology alone. Systematic comparative analysis; clinical and laboratory analyses, especially 18F-FDG-PET/CT; and responses to drug treatment are therefore important parameters for distinguishing between these two diseases.


Assuntos
Hiperplasia do Linfonodo Gigante/sangue , Imunoglobulina G/sangue , Interleucina-6/sangue , Linfadenopatia/sangue , Adulto , Idoso , Proteína C-Reativa/análise , Hiperplasia do Linfonodo Gigante/diagnóstico por imagem , Hiperplasia do Linfonodo Gigante/imunologia , Diagnóstico Diferencial , Humanos , Imunoglobulina G/imunologia , Testes Imunológicos/métodos , Linfadenopatia/diagnóstico por imagem , Linfadenopatia/imunologia , Pessoa de Meia-Idade , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons/métodos , Estudos Retrospectivos , Sensibilidade e Especificidade , Tomografia Computadorizada por Raios X/métodos
8.
Rinsho Ketsueki ; 59(8): 991-996, 2018.
Artigo em Japonês | MEDLINE | ID: mdl-30185716

RESUMO

TAFRO syndrome is characterized by thrombocytopenia with unknown etiology. The assessment of immature platelet fraction (IPF) is useful for differential diagnoses that include thrombocytopenia. However, the significance of IPF in cases of TAFRO syndrome remains to be reported. We present a case of TAFRO syndrome wherein the patient demonstrated a marked increase in IPF without thrombocytopenia, which offers vital information concerning TAFRO diagnosis and the serial measurements of IPF during treatment. A 65-year-old man presenting with fever was admitted to our hospital. He exhibited mild splenomegaly and lymphadenopathy, as well as rapidly worsening renal failure and fluid retention. These indications prompted the initiation of corticosteroid therapy. A normal platelet count and aberrantly high IPF implied abnormal thrombopoiesis, and subsequent bone-marrow findings suggested TAFRO syndrome. The platelet counts started to decrease following the corticosteroid therapy, but the treatment refractoriness prompted the urgent administration of rituximab. Thereafter, the platelet count nadir remained for approximately one month, whereas the decreasing IPF trend preceded platelet recovery. In the present case, a high pre-treatment IPF was demonstrated before the emergence of thrombocytopenia, and a decreasing trend of IPF was observed before platelet recovery during treatment. Therefore, serial IPF measurements could be useful for the early diagnosis and prognostication of TAFRO syndrome.


Assuntos
Plaquetas/citologia , Hiperplasia do Linfonodo Gigante/diagnóstico , Idoso , Hiperplasia do Linfonodo Gigante/sangue , Diagnóstico Diferencial , Humanos , Masculino , Contagem de Plaquetas , Trombocitopenia
9.
Pediatrics ; 142(3)2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-30139808

RESUMO

Multicentric Castleman disease (MCD) is a rare entity that, unlike unicentric Castleman disease, involves generalized polyclonal lymphoproliferation, systemic inflammation, and multiple-organ system failure resulting from proinflammatory hypercytokinemia, including, in particular, interleukin-6. A subset of MCD is caused by human herpesvirus-8 (HHV-8), although the etiology for HHV-8-negative, idiopathic MCD (iMCD) cases is unknown at present. Recently, a consensus was reached on the diagnostic criteria for iMCD to aid in diagnosis, recognize mimics, and initiate prompt treatment. Pediatric iMCD remains particularly rare, and differentiation from MCD mimics in children presenting with systemic inflammation and lymphoproliferation is a challenge. We report on a young boy who presented with a HHV-8-negative, iMCD-like phenotype and was found to suffer from the monogenic disorder deficiency of adenosine deaminase 2 (DADA2), which is caused by loss-of-function mutations in CECR1 DADA2 prototypic features include early-onset ischemic and hemorrhagic strokes, livedoid rash, systemic inflammation, and polyarteritis nodosa vasculopathy, but marked clinical heterogeneity has been observed. Our patient's presentation remains unique, with predominant systemic inflammation, lymphoproliferation, and polyclonal hypergammaglobulinemia but without apparent immunodeficiency. On the basis of the iMCD-like phenotype with elevated interleukin-6 expression, treatment with tocilizumab was initiated, resulting in immediate normalization of clinical and biochemical parameters. In conclusion, iMCD and DADA2 should be considered in the differential diagnosis of children presenting with systemic inflammation and lymphoproliferation. We describe the first case of DADA2 that mimics the clinicopathologic features of iMCD, and our report extends the clinical spectrum of DADA2 to include predominant immune activation and lymphoproliferation.


Assuntos
Adenosina Desaminase/deficiência , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/diagnóstico , Peptídeos e Proteínas de Sinalização Intercelular/deficiência , Pré-Escolar , Diagnóstico Diferencial , Humanos , Masculino
11.
J Cancer Res Clin Oncol ; 144(7): 1265-1277, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29736622

RESUMO

PURPOSE: HIV negative Castleman's disease has been reported as a group of poorly understood lymphoproliferative disorder, and we want to explore the clinical feature and prognosis factors of CD. METHODS: We retrospectively collected the clinical information of 71 CD patients without HIV infection diagnosed in the first affiliated hospital of Zhengzhou university. RESULTS: Different clinical classifications, including 35 patients (49.30%) with unicentric Castleman disease and 36 (50.7%) with multicentric Castleman disease, has their specific features compared with each other and unfavorable risk factors calculated by the univariate analysis. As for all of CD patients without HIV infection, there were 7 significant risk factors identified by the results of log-rank test, including clinical complaint, edema (hydrothorax, ascites, pelvic effusion), fatigue, anemia, hypoproteinemia and elevated serum ß2-MG. Then, we created a Cox regression model of these clinical and statistic significant factors which indicated hypoproteinemia was an independent poor prognosis factors of CD in both univariate and multivariate analysis. CONCLUSIONS: Our study emphasized the distinction of clinical characteristics between UCD and MCD and the importance of different poor risk factors of different clinical classifications which may directed more precise and appropriate treatment strategy.


Assuntos
Biomarcadores/sangue , Hiperplasia do Linfonodo Gigante/sangue , Hipoproteinemia/etiologia , Adolescente , Adulto , Idoso , Hiperplasia do Linfonodo Gigante/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Adulto Jovem
13.
Am J Hematol ; 93(7): 902-912, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29675946

RESUMO

Human Herpesvirus-8 (HHV-8)-negative/idiopathic multicentric Castleman disease (iMCD) is a poorly understood disease involving polyclonal lymphoproliferation with dysmorphic germinal centers, constitutional symptoms, and multi-organ failure. Patients can experience thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction, organomegaly, and normal immunoglobulin levels, - iMCD-TAFRO. Others experience thrombocytosis, milder effusions, and hypergammaglobulinemia, -iMCD-Not Otherwise Specified (iMCD-NOS). Though the etiology is unknown in both subtypes, iMCD symptoms and disease progression are believed to be driven by a cytokine storm, often including interleukin-6 (IL-6). However, approximately two-thirds of patients do not respond to anti-IL-6 therapy; alternative drivers and signaling pathways are not known for anti-IL-6 nonresponders. To identify potential mediators of iMCD pathogenesis, we quantified 1129 proteins in 13 plasma samples from six iMCD patients during flare and remission. The acute phase reactant NPS-PLA2 was the only significantly increased protein (P = .017); chemokines and complement were significantly enriched pathways. Chemokines represented the greatest proportion of upregulated cytokines, suggesting that iMCD involves a chemokine storm. The chemokine CXCL13, which is essential in homing B cells to germinal centers, was the most upregulated cytokine across all patients (log2 fold-change = 3.22). Expression of CXCL13 was also significantly increased in iMCD lymph node germinal centers compared to controls in a stromal meshwork pattern. We observed distinct proteomic profiles between the two iMCD-TAFRO patients, who both failed anti-IL-6-therapy, and the four iMCD-NOS patients, in whom all three treated with anti-IL-6-therapy responded, suggesting that differing mechanisms may exist. This study reveals proteomic differences between flare and remission and the potential to molecularly define iMCD subgroups.


Assuntos
Hiperplasia do Linfonodo Gigante/etiologia , Quimiocinas/metabolismo , Plasma/química , Proteômica/métodos , Adulto , Anticorpos Monoclonais/uso terapêutico , Hiperplasia do Linfonodo Gigante/sangue , Quimiocina CXCL13/metabolismo , Feminino , Humanos , Interleucina-6/imunologia , Masculino , Pessoa de Meia-Idade , Regulação para Cima
15.
Hematol Oncol Clin North Am ; 32(1): 11-21, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29157613

RESUMO

Castleman disease (CD) describes a group of heterogeneous disorders with common lymph node histopathologic features, including atrophic or hyperplastic germinal centers, prominent follicular dendritic cells, hypervascularization, polyclonal lymphoproliferation, and/or polytypic plasmacytosis. The cause and pathogenesis of the four subtypes of CD (unicentric CD; human herpesvirus-8-associated multicentric CD; polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes [POEMS]-associated multicentric CD; and idiopathic multicentric CD) vary considerably. This article provides a summary of our current understanding of the cause, cell types, signaling pathways, and effector cytokines implicated in the pathogenesis of each subtype.


Assuntos
Hiperplasia do Linfonodo Gigante , Citocinas/sangue , Infecções por Herpesviridae , Herpesvirus Humano 8/metabolismo , Linfonodos , Proteínas de Neoplasias/sangue , Transdução de Sinais , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/fisiopatologia , Hiperplasia do Linfonodo Gigante/virologia , Infecções por Herpesviridae/sangue , Infecções por Herpesviridae/fisiopatologia , Humanos , Linfonodos/metabolismo , Linfonodos/fisiopatologia , Linfonodos/virologia
16.
Hematol Oncol Clin North Am ; 32(1): 23-36, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29157617

RESUMO

Since its discovery, improvements in treating Castleman disease and its variants have centered on interleukin-6 (IL-6). IL-6 was discovered from T-cell factors (BCDF or BSF-2), which induced B-cell maturation. Most symptoms of the plasma cell variant of Castleman disease are linked to the hyperfunction of IL-6, constitutively produced in the affected lymph nodes (1989), suggesting IL-6 is key in the pathogenesis of multicentric Castleman disease (MCD). The results of several studies have shown that most MCD symptoms and abnormal laboratory results are improved by anti-IL-6 MCD treatments, such as tocilizumab, a humanized anti-IL-6 receptor antibody, and siltuximab, an anti-IL-6 antibody.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Hiperplasia do Linfonodo Gigante , Interleucina-6/sangue , Linfonodos/metabolismo , Proteínas de Neoplasias/sangue , Plasmócitos/metabolismo , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Humanos , Interleucina-6/antagonistas & inibidores , Proteínas de Neoplasias/antagonistas & inibidores
17.
Hematol Oncol Clin North Am ; 32(1): 89-106, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29157622

RESUMO

Important progress has been made in the treatment of idiopathic multicentric Castleman disease (iMCD) with the introduction of interleukin-6 targeting monoclonal antibodies. This article describes the clinical results obtained with different treatment modalities and uses this evidence to provide treatment guidelines for the practicing clinician. Much is still to be learned about the pathophysiology of iMCD and further research is urgently needed to develop novel and curative treatment approaches for all patients.


Assuntos
Antineoplásicos Imunológicos/uso terapêutico , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Interleucina-6/antagonistas & inibidores , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/patologia , Humanos , Interleucina-6/sangue , Guias de Prática Clínica como Assunto
19.
Ann Clin Lab Sci ; 47(3): 315-318, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28667033

RESUMO

BACKGROUND: Platelet refractoriness or lack of platelet increase after platelet transfusion is seen in patients receiving chronic platelet transfusion support. Antibodies may develop against human platelet antigens (HPA) and/or against HLA class I antigens. Crossmatch (XM) compatible platelets or HLA-identical or HLA-compatible platelets are typically used to manage transfusion refractoriness. We aimed to determine if percent calculated Panel Reactive Antibody (% cPRA) against class I HLA antigens could predict percent positive platelet XM when looking for compatible transfusion products. METHODS: A retrospective review of all platelet XM performed at our institution between 2008-2012 was performed, and patient characteristics recorded. For each patient, the percentage of all positive platelet XM performed was calculated and compared with the corresponding % cPRA levels against class I HLA antigens. RESULTS: Mean and median % positive platelet XM for all 50 patients tested in the period 2008-2012 were 61% and 60% (range 0-100%), respectively. Mean and median % cPRA levels were 66% and 68% (range 0-100%), respectively. No correlation was seen between age, sex, race, or diagnosis and positive platelet XM results. CONCLUSION: The results of our study indicate that the % cPRA correlates well with the % positive platelet XM. Thus, a higher % cPRA alerts the blood bank that additional platelets will be required for XM and/or that it would be beneficial to request HLA-identical or compatible units.


Assuntos
Tipagem e Reações Cruzadas Sanguíneas , Plaquetas/imunologia , Adulto , Idoso , Transfusão de Sangue , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/imunologia , Feminino , Fibrose/sangue , Fibrose/imunologia , Antígenos de Histocompatibilidade Classe I , Humanos , Linfoma Folicular/sangue , Linfoma Folicular/imunologia , Linfoma Difuso de Grandes Células B/sangue , Linfoma Difuso de Grandes Células B/imunologia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/sangue , Mieloma Múltiplo/imunologia , Estudos Retrospectivos
20.
Sci Rep ; 7: 42316, 2017 02 13.
Artigo em Inglês | MEDLINE | ID: mdl-28205564

RESUMO

Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder. It is characterized by inflammatory symptoms, and interleukin (IL)-6 contributes to the disease pathogenesis. Human herpesvirus 8 (HHV-8) often drives hypercytokinemia in MCD, although the etiology of HHV-8-negative MCD is idiopathic (iMCD). A distinct subtype of iMCD that shares a constellation of clinical features including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been reported as TAFRO-iMCD, however the differences in cytokine profiles between TAFRO-iMCD and iMCD have not been established. We retrospectively compared levels of serum interferon γ-induced protein 10 kDa (IP-10), platelet-derived growth factor (PDGF)-AA, interleukin (IL)-10, and other cytokines between 11 cases of TAFRO-iMCD, 6 cases of plasma cell type iMCD, and 21 healthy controls. During flare-ups, patients with TAFRO-iMCD had significantly higher serum IP-10 and tended to have lower PDGF-AA levels than the other 2 groups. In addition, serum IL-10, IL-23, and vascular endothelial growth factor-A were elevated in both TAFRO-iMCD and iMCD. Elevated serum IP-10 is associated with inflammatory diseases including infectious diseases. There was a strong correlation between high serum IP-10 and the presence of TAFRO-iMCD, suggesting that IP-10 might be involved in the pathogenesis of TAFRO-iMCD.


Assuntos
Hiperplasia do Linfonodo Gigante/sangue , Quimiocina CXCL10/sangue , Adulto , Idoso , Estudos de Casos e Controles , Hiperplasia do Linfonodo Gigante/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome
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